Cicatricial pemphigoid

What is cicatricial pemphigoid?

Cicatricial pemphigoid is an autoimmune disease that is characterised by blistering lesions on mucous membranes. It is also called benign mucous membrane pemphigoid or oral pemphigoid. Areas commonly involved are the oral mucosa (lining of the mouth) and conjunctiva (mucous membrane that coats the inner surface of the eyelids and the outer surface of the eye). Other areas that may be affected include the nostrils, oesophagus, trachea and genitals. Sometimes the skin may also be involved where blistering lesions can be found on the face, neck and scalp.

Brunsting Perry cicatricial pemphigoid is a rare variant in which localised crops of recurrent blisters arise within urticarial plaques, usually on the head and neck. The blisters may burst resulting in blood-crusted plaques and scars.

Who gets cicatricial pemphigoid?

Cicatricial pemphigoid is predominantly a disease of the elderly with a peak incidence at around 70 years. However, childhood cases have been reported. It appears to be twice as common in women than men.

What are the signs and symptoms of cicatricial pemphigoid?

Site

Features

Eye

Sensation of grittiness or pain

Conjunctivitis

Lesions form, erode and heal to leave scar tissue

May lead to impaired vision or blindness

Mouth

Blisters form first on the gums near the teeth

Palate, tongue, lips, buccal mucosa, floor of the mouth and throat may be affected

Painful and make it difficult to eat

Lesions occurring in the throat (oesophagus, trachea and larynx) can become life-threatening

Skin

Blisters on the skin develop in 25-30% of patients

May be itchy

Bleeding may occur if traumatised

Nose

Nose bleeds after blowing the nose

Crusting causing discomfort

Genitals

Painful blisters and erosions on the clitoris, labia, shaft of the penis, perianal area

What causes cicatricial pemphigoid?

Cicatricial pemphigoid is an autoimmune blistering disease, which basically means that an individual’s immune systems starts reacting against his or her own tissue. In this particular instance autoantibodies react with proteins found in mucous membranes and skin tissue resulting in blistering lesions. The binding site appears to be within the anchoring filaments that help the epidermis (outside layer of skin) stick to the dermis (inner layer of skin).

About IPPF

The International Pemphigus & Pemphigoid Foundation’s most important objectives are to provide patients and doctors worldwide with information about pemphigus and pemphigoid, and to provide patients and their caregivers much needed comfort and support so they can continue to live active, productive lives.Read more »